Dermatitis herpetiformis

What it is

From: Orphanet

A chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. It may also appear as a consequence of gluten intolerance.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Abnormal blistering of the skin · Very frequent (99-80%)

The presence of one or more bullae on the skin, defined as fluid-filled blisters more than 5 mm in diameter with thin walls.

Autoimmunity · Very frequent (99-80%)

The occurrence of an immune reaction against the organism's own cells or tissues.

Erythema · Very frequent (99-80%)

Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.

Gluten intolerance · Very frequent (99-80%)

A detrimental reaction to the presence of gluten in food, which may include abdominal pain, fatigue, headaches and paresthesia, or celiac disease.

Macule · Very frequent (99-80%)

A flat, distinct, discolored area of skin less than 1 cm wide that does not involve any change in the thickness or texture of the skin.

Malabsorption · Very frequent (99-80%)

Impaired ability to absorb one or more nutrients from the intestine.

Microcytic anemia · Very frequent (99-80%)

A kind of anemia in which the volume of the red blood cells is reduced.

Pruritus · Very frequent (99-80%)

Pruritus is an itch or a sensation that makes a person want to scratch. This term refers to an abnormally increased disposition to experience pruritus.

Recurrent fractures · Very frequent (99-80%)

The repeated occurrence of bone fractures (implying an abnormally increased tendency for fracture).

Skin vesicle · Very frequent (99-80%)

A circumscribed, fluid-containing, epidermal elevation less than 10mm in diameter at the widest point that (i) Contain serous exudates or serum mixed with blood or pus; (ii) Are discrete, grouped, irregularly distributed, or linear as in Rhus dermatitis; (iii) Are short-lived. Vesicles may break spontaneously or evolve into bullae by enlarging or coalescing with other vesicles.

Urticaria · Very frequent (99-80%)

Raised, well-circumscribed areas of erythema and edema involving the dermis and epidermis. Urticaria is intensely pruritic, and blanches completely with pressure.

Anti-endomysial antibody positivity · Frequent (79-30%)

The presence of autoantibodies (immunoglobulins) in the blood circulation that react against endomysial tissue transglutaminase 2 (tTG2).

Anti-epidermal transglutaminase antibody positivity · Frequent (79-30%)

The presence of autoantibodies (immunoglobulins) in the blood circulation that react against epidermal transglutaminase.

Anti-reticulin antibody positivity · Frequent (79-30%)

The presence of autoantibodies (immunoglobulins) in the serum that react against reticulin.

Other findings in the same source

From: Orphanet

Additional reported features include Anti-transglutaminase 6 antibody (Frequent (79-30%)); Anti-type VII collagen antibody (Frequent (79-30%)); Antigliadin antibody positivity (Frequent (79-30%)); Eczematoid dermatitis (Frequent (79-30%)); Abnormality of the thyroid gland (Occasional (29-5%)); Bone pain (Occasional (29-5%)); Delayed eruption of teeth (Occasional (29-5%)); Dental enamel pits (Occasional (29-5%)); Edema (Occasional (29-5%)); Lichenification (Occasional (29-5%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

All ages

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: 1-5 / 10 000; Europe; Value and class. Annual incidence: 1-9 / 1 000 000; Italy; Value and class. Annual incidence: 1-9 / 100 000; Ireland; Value and class. Point prevalence: 1-5 / 10 000; Ireland; Value and class.

Which doctor should you see?

The suggested department for discussing Dermatitis herpetiformis is Dermatology, with a dermatologist as the relevant type of clinician. Dermatologist; paediatric services for children as appropriate.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Which features of the skin, hair or nails distinguish the possibilities?
• Would photographs over time help document the changes?
• What should be expected from treatment, and how will irritation or other adverse effects be managed?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Dermatitis herpetiformis. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.